Abstract: The patient, a 25-year-old woman, was seen at our hospital 6 years ago, complaining of weakness and stiffness in the left side of the body and left limbs for 1⁺ years. Physical examination revealed atrophy of the upper and lower limbs on the left side. Neurological examination showed increased muscle tone in the left-side body and limbs, bradykinesia, decreased muscle strength in the left-side body and limbs, and positive Hoffman's sign in the left limbs. Laboratory tests, including alpha fetoprotein (AFP), β-human chorionic gonadotropin (HCG) and cerebrospinal fluid examination, did not reveal any abnormal results. Head MRI showed right cerebral hemiatrophy. Head and neck CT angiography did not show obvious abnormality. According to the medical history and examination results, diagnosis of hemiparkinsonism-hemiatrophy syndrome was made. Through close follow-up for 6 years, we noticed that the parkinsonism remained unilateral and stable, and the contralateral cerebral hemiatrophy did not show obvious progress. However, brain perfusion MRI showed hypoperfusion of the right basal ganglia. The discovery of hypoperfusion in the basal ganglia may help explore the etiology of hemiparkinsonism-hemiatrophy syndrome.